Diabetic nephropathy is a common and serious complication in diabetic patients. Renal diseases other than diabetic nephropathy (non-diabetic nephropathy) can occur in diabetic patients with nephrosis. The presence of non-diabetic nephropathy is noted in type 2 diabetes patients, but no data exists for type 1 diabetes. In this report we describe the case of a 15-year-old girl with type 1 diabetes mellitus, who presented with an acute elevation of urinary microalbumin excretion, general edema, and liver enzyme elevation. She had shown microalbuminuria about 3 years earlier, as well as an uncontrolled hemoglobin A1c level, but she had no diabetic retinopathy and neuropathy. A renal biopsy was conducted, and she was diagnosed with primary focal segmental glomerulosclerosis. She was treated with corticosteroids and an angiotensin converting enzyme inhibitor.
Adolescent ; Adrenal Cortex Hormones ; Biopsy ; Diabetes Mellitus, Type 1 ; Diabetic Nephropathies ; Diabetic Retinopathy ; Edema ; Glomerulosclerosis, Focal Segmental ; Hemoglobins ; Humans ; Liver ; Nephrosis ; Peptidyl-Dipeptidase A

PURPOSE: Gastric variceal bleeding in cirrhotic patient is an emergent, life threatening disease of which the adequate treatment is still in dispute. The periesophagogastric devascularization (PD) would be the alternative operative procedure. The aim of this study is to evaluate the safety and long-term results of PD for the gastric fundal variceal bleeding. METHODS: A retrospective analysis was made of fifty-two patients with gastric variceal bleeding who underwent PD with or without fundectomy from August 1994 to March 2006. We evaluated the clinical characteristics, operative morbidity, mortality and long-term follow up results. RESULTS: The operative morbidity was 34.9% and overall mortality was 17.3%. The Child-Pugh classification at operation, the number of previous varix bleeding episode, the success of preoperative intervention, splenectomy, esophageal transection, and ongoing bleeding at operation had a significant effect on postoperative mortality. Most common cause of postoperative death was hepatic failure. Child-Pugh classification and esophageal transection were significant risk factor for postoperative mortality comparing before and after year 2001. During the mean follow-up period of 33.58+/-27.08 months, there was no recurrent bleeding from gastric varices. The cumulative 5-year survival rate was 64.1%. 0f 14 patients deceased, 6 patients died of hepatocellular carcinoma. Not only there was no death caused by recurrent variceal bleeding, but there was no recurrent gastric variceal bleeding. Hepatic functional reserve and concomitant hepatocellular carcinoma were the most important prognostic factors in the long term survival by multiple regression analysis. CONCLUSION: We concluded that PD reached both low postoperative mortality and recurrent bleeding rate with good long-term survival. So it could be one of the most effective treatment for the gastric fundal variceal bleeding.
Carcinoma, Hepatocellular ; Classification ; Dissent and Disputes ; Esophageal and Gastric Varices* ; Follow-Up Studies ; Hemorrhage ; Humans ; Liver Cirrhosis ; Liver Failure ; Mortality ; Retrospective Studies ; Risk Factors ; Splenectomy ; Surgical Procedures, Operative ; Survival Rate ; Varicose Veins

OBJECTIVE: The perioperative management of antithrombotic therapy is often challenging and it requires a fine balance between the risk of hemorrhage and thrombosis. We aimed to evaluate the antithrombotic management for moderate to high risk patients in real world setting. METHODS: Among the patients who were consulted to the neurologist for the evaluation of perioperative risk from 2010 to 2012, patients undergoing moderate to high risk surgery and taking antithrombotics within 30 days were identified. We analyzed the timing of discontinuation and reinitiation of antithrombotic drugs before or after surgery as well as the status of bridging therapy. In addition, the conformity with the guideline suggested by American College of Chest Physicians was assessed. The rate of thromboembolic event and major hemorrhage were also investigated. RESULTS: A total of 329 patients were included. The concordance rate of warfarin stop and restart time with guideline was 23.4% and 10.3%, respectively. Continuing aspirin in patients undergoing coronary artery bypass surgery or non-cardiac surgery in patients with high risk for cardiovascular events were 59.2% and 2.6%, respectively. Bridging therapy was adopted in 92.9% and 81.2% in patients who had received anticoagulant before surgery and who were at high and low risk thromboembolism, respectively. In entire cohorts, 30-day incidence of major bleeding and thromboembolic event were 31.9% and 3.0%. Co-morbid renal disease were shown as independent predictor for major bleeding (adjusted OR 2.65. 95% CI 1.33-5.28). CONCLUSION: The concordance rate with guideline regarding perioperative antithrombotic use was low and bridging therapy was prevalent in patients undergoing moderate to high risk surgery.
Anticoagulants ; Aspirin ; Cohort Studies ; Coronary Artery Bypass ; Hemorrhage ; Humans ; Incidence ; Thorax ; Thromboembolism ; Thrombosis ; Warfarin

Fraley's syndrome is a disorder of vascular impression on the renal infundibulum which is associated with unexplained disabling flank pain, painless microscopic hematuria, and urinary tract infection. We experienced one case of Fraley's syndrome in a 12-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures.
Child ; Female ; Flank Pain ; Hematuria* ; Humans ; Urinary Tract Infections

PURPOSE: The aims of this study are to evaluate psychological impact and quality of life according to the cancer diagnosis and mutation status in Korean families with BRCA mutations. MATERIALS AND METHODS: Seventeen affected carriers (AC), 16 unaffected carriers (UC) and 13 healthy non carriers (NC) from 13 BRCA mutation families were included in the study. Outcomes were compared with regard to depression (Beck Depression Inventory), anxiety (State-Trait Anxiety Inventory, STAI), optimism (Reevaluation of the Life Orientation test, LOT-R), knowledge of hereditary ovarian cancer, and quality of life (QoL) (SF-36v2 Health Survey, physical component score [PCS], mental component score [MCS]) among three groups. RESULT: Level of depression, optimism, and PCS were similar in AC, UC, and NC. Anxiety score was elevated in all three groups. MCS was significantly low in AC than in UC and NC (P =0.009, P =0.017). Knowledge of hereditary breast and ovarian cancer was high in AC than NC (P =0.001). MCS was significantly related to whether patient was affected by cancer (P =0.043) and has occupation (P = 0.008) or not in multivariable analysis. CONCLUSION: From this cross sectional study, psychological adverse effect was not related to the carrier status of BRCA mutation. Elevated anxiety in BRCA family members was observed but, independent to affection and the type of genetic mutation. AC showed low mental QoL. Further effort to understand psychological impact and QoL of genetic testing in BRCA family members is required for follow-up in clinical aspects.
Anxiety ; Breast ; Depression ; Genetic Counseling ; Genetic Testing ; Health Surveys ; Humans ; Occupations ; Orientation ; Ovarian Neoplasms ; Quality of Life

Multiple myeloma (MM) is a malignant disease caused by an abnormal proliferation of plasma cells, of which the prognostic factors include chromosomal abnormality, β-2 microglobulin, and albumin. Recently, the term chromothripsis has emerged, which is the massive but highly localized chromosomal rearrangement in response to a one-step catastrophic event. Many studies have shown an association of chromothripsis with the prognosis in several cancers; however, few studies have investigated it in MM. Here, we studied the association between chromothripsis-like patterns and treatment resistance or prognosis. First, we analyzed nine MM cell lines (U266, MM.1S, RPMI8226, KMS-11, KMS-12-BM, KMS-12-PE, KMS-28-BM, KMS-28-PE, and NCI-H929) and bone marrow samples of four patients who were diagnosed with MM by next-generation sequencing-based copy number variation analysis. The frequency of the chromothripsis-like pattern was observed in seven cell lines. We analyzed the treatment-induced chromothripsis-like patterns in KMS-12-BM and KMS-12-PE cells. As a result, breakpoints and chromothripsis-like patterns were increased after drug treatment in the relatively resistant KMS-12-BM. We further analyzed the patients’ results according to the therapeutic response, which was divided into sensitive and resistant, as suggested by the International Myeloma Working Group. The chromothripsis-like pattern was more frequently observed in the resistant group. In the sensitive group, the frequency of the chromothripsis-like pattern decreased after treatment, whereas the resistant group showed increased chromothripsis-like patterns after the treatment. These results suggest that the chromothripsis-like pattern is associated with treatment response in MM.
Bone Marrow ; Cell Line ; Chromosome Aberrations ; Drug Resistance ; Humans ; Multiple Myeloma* ; Plasma Cells ; Prognosis

PURPOSE: This study was done to analyze how a death preparation education program have the effects on awareness of hospice palliative care and withdrawing life sustaining treatment in older adults. METHODS: This study employed a non-equivalent control group design among quasi-experimental designs. The experimental group was comprised with 35 adults and the control group with 40 adults. The death preparation program consisted of five two-hour sessions and was administered once a week for five straight weeks. Data were analyzed by descriptive statistics, t-test, chi2 test, Fisher's exact test, and ANCOVA using SPSS version 18.0. RESULTS: The mean age of the participants was 66.2 years. A significant difference between the experimental and control groups was observed in withdrawing life sustaining treatment (F=3.380, P=0.040). However, no significant difference was found in awareness of hospice palliative care (F=0.163, P=0.850). CONCLUSION: The study results indicate that death preparation training could positively affect people's awareness of withdrawing life sustaining treatment. More studies should be conducted to explore effects of death preparation education for all ages and help people better understand hospice palliative care.
Adult ; Education* ; Hospices* ; Humans ; Life Support Care ; Palliative Care*

BACKGROUND: A range of well characterized materials are needed for validating the performance of hepatitis B surface antigen (HBsAg) immunoassays. These materials are purchased currently from overseas manufacturers at a high cost and with limited quantity. This study was conducted to establish an HBsAg low titer performance panel for use as a national standard for validation of HBsAg immunoassays in Korea. METHODS: 476 plasma units reactive on blood donor screening were collected HBsAg was tested using 3 enzyme immunoassays (EIA) and 1 chemiluminescence immunoassay (CIA). Units reactive on the CIA assay or on 2 or more immunoassays were subjected to hepatitis B virus (HBV) DNA quantification, HBV genotyping and subtyping. Units reactive on HBV DNA quantification were confirmed for HBsAg by neutralization. Candidates for the panel were subjected to a collaborative study performed at 7 laboratories using 7 immunoassays. RESULTS: Eleven HBsAg positive units were selected for the low titer performance panel based on HBsAg immunoassay, HBV DNA quantification, HBV genotyping and subtyping results. The range of the HBsAg concentration of the panel members was 0.05~1.28 IU/mL. Two HBsAg negative units were also included as negative controls. CONCLUSION: As a result of this study, a low titer performance panel [KFDA standard (08/028); HBsAg low titer performance panel (BTRL HBV/LP)] for validation of HBsAg immunoassays has been established as a Korean national standard. Use of this panel will improve performance assessment of HBsAg immunoassays. Because the performance of immunoassays cannot be assessed properly with a limited number of panels, continuous efforts are needed to develop a range of performance panels.
Blood Donors ; DNA ; Hepatitis ; Hepatitis B ; Hepatitis B Surface Antigens ; Hepatitis B virus ; Humans ; Immunoassay ; Immunoenzyme Techniques ; Luminescence ; Mass Screening ; Plasma

BACKGROUND: Thoracic paravertebral blockade (TPVB) can be effective in many clinical settings. However, many clinicians are reluctant to use this technique due to its adverse effects such as pneumothorax. The aim of this study was estimate the appropriate depth and width for safe needle positioning. METHODS: The depth from skin to the paravertebral space (PVS) and the width from the spinous process (SP) to the PVS and the lateral tip of the transverse process (LTTP) were measured in all available sections from 84 patients examined by chest computed tomography (CT). The correlation between age, gender, body mass index (BMI) and each measured value was analyzed. RESULTS: All 828 sections from the 84 patients aged between 19 and 65 years old were evaluated. The minimum and maximum values of the mean depth from the skin to PVS were 40.3-52.4 mm in men and 41.2-55.9 mm in women. The minimum and maximum values of the mean width from the SP to LTTP were 29.2-34.6 mm in men and 23.7-31.0 mm in women. At every thoracic level, the depth to PVS was similar in both genders but the width from the SP to the NEP in men was significantly higher than in women. Weight significantly influenced the width from the SP to LTTP in men but height and BMI did not. Also weight and BMI significantly influenced the depth from skin to PVS in almost all of the thoracic region at both sex, but height did not influence the depth from skin to PVS in both sex. CONCLUSIONS: We report clinically useful estimates that may be used to help avoid side effects such as pneumothorax and achieve a successful thoracic paravertebral blockade.
Aged ; Body Mass Index ; Female ; Humans ; Male ; Needles* ; Pneumothorax ; Skin ; Thorax

Interleukin-10 (IL10) plays an important role in initiating and maintaining an appropriate immune response to non-Hodgkin lymphoma (NHL). Previous studies have revealed that the transcription of IL10 mRNA and its protein expression may be infl uenced by several single-nucleotide polymorphisms in the promoter and intron regions, including rs1800896, rs1800871, and rs1800872. However, the impact of polymorphisms of the IL10 gene on NHL prognosis has not been fully elucidated. Here, we investigated the association between IL10 polymorphisms and NHL prognosis. This study involved 112 NHL patients treated at the National Cancer Center, Korea. The median age was 57 years, and 70 patients (62.5%) were men. Clinical characteristics, including age, performance status, stage, and extra-nodal involvement, as well as cell lineage and International Prognostic Index (IPI), were evaluated. A total of four polymorphisms in IL10 with heterozygous alleles were analyzed for hazard ratios of overall survival (OS) and progression-free survival (PFS) using Cox proportional hazards regression analysis. Diffuse large B-cell lymphoma was the most common histologic type (n = 83), followed by T-cell lymphoma (n = 18), mantle cell lymphoma (n = 6), and others (n = 5). Cell lineage, IPI, and extra-nodal involvement were predictors of prognosis. In the additive genetic model results for each IL10 polymorphism, the rs1800871 and rs1800872 polymorphisms represented a marginal association with OS (p = 0.09 and p = 0.06) and PFS (p = 0.05 and p = 0.08) in B-cell lymphoma patients treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). These findings suggest that IL10 polymorphisms might be prognostic indicators for patients with B-cell NHL treated with R-CHOP.
Alleles ; B-Lymphocytes* ; Cell Lineage ; Cyclophosphamide ; Disease-Free Survival ; Doxorubicin ; Humans ; Interleukin-10* ; Introns ; Korea ; Lymphoma, B-Cell* ; Lymphoma, Mantle-Cell ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell ; Male ; Models, Genetic ; Polymorphism, Single Nucleotide ; Prednisone ; Prognosis* ; Rituximab ; RNA, Messenger ; Vincristine